Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria.
Document Type
Article
Publication Date
7-1-2021
Identifier
DOI: 10.1093/rheumatology/keaa757
Abstract
OBJECTIVE: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population.
METHODS: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations. We analysed patterns of symptoms, diagnostic workup, and applied the 2016 ACR/EULAR classification criteria.
RESULTS: We identified 300 children with Sjögren syndrome. The majority of patients n = 232 (77%) did not meet 2016 ACR/EULAR classification criteria, but n = 110 (37%) did not have sufficient testing done to even possibly achieve the score necessary to meet criteria. Even among those children with all criteria items tested, only 36% met criteria. The most common non-sicca symptoms were arthralgia [n = 161 (54%)] and parotitis [n = 140 (47%)] with parotitis inversely correlating with age.
CONCLUSION: Sjögren syndrome in children can present at any age. Recurrent or persistent parotitis and arthralgias are common symptoms that should prompt clinicians to consider the possibility of Sjögren syndrome. The majority of children diagnosed with Sjögren syndromes did not meet 2016 ACR/EULAR classification criteria. Comprehensive diagnostic testing from the 2016 ACR/EULAR criteria are not universally performed. This may lead to under-recognition and emphasizes a need for further research including creation of paediatric-specific classification criteria.
Journal Title
Rheumatology (Oxford, England)
Volume
60
Issue
7
First Page
3144
Last Page
3155
Keywords
Sjögren Syndrome; childhood Sjögren syndrome; pediatric rheumatology; recurrent parotitis
Recommended Citation
Basiaga ML, Stern SM, Mehta JJ, et al. Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria. Rheumatology (Oxford). 2021;60(7):3144-3155. doi:10.1093/rheumatology/keaa757
COinS
